BMC Ear, Nose and Throat Disorders最新文献

Background: The light cupula is a condition wherein the cupula of the semicircular canal has a lower specific gravity than its surrounding endolymph. It is characterized by a persistent geotropic direction-changing positional nystagmus in the supine head-roll test, and the identification of a null plane with slight head-turning to either side.

Case presentation: This study describes a case of recurring light cupula that occurred alternately on both sides. At the first episode, a null plane was identified on the right side, which led to the diagnosis of a light cupula on the right side. At the second episode, a null plane was identified on the left side, leading to the diagnosis of a light cupula on the left side.

Conclusion: This is the first case report of recurring light cupula alternately involving both sides. Although the pathophysiology is not entirely understood yet, the light cupula should be considered as one of causes of recurrent positional vertigo.

Background: Diagnostic imaging of the head is used with increasing frequency, and often includes the paranasal sinuses, where incidental opacifications are found. To determine the clinical relevance of such findings can be challenging, and for the patient such incidental findings can give rise to concern if they are over-reported. Studies of incidental findings in the paranasal sinuses have been conducted mostly in patients referred for diagnostic imaging, hence the prevalence in the general population is not known. The purpose of this study was to determine the prevalence and size of incidental opacification in the paranasal sinuses in a non-selected adult population using magnetic resonance imaging (MRI) without medical indication, and to relate the results to sex and season.

Methods: Randomly and independent of medical history, 982 participants (518 women) with a mean age of 58.5 years (range, 50-66) underwent MRI of the head as part of a large public health survey in Norway. The MRIs included 3D T1 weighted volume data and 2D axial T2 weighted image (WI). Opacifications, indicating mucosal thickenings, polyps, retention cysts, or fluid, were recorded if measuring more than 1 mm.

Results: Opacifications were found in 66% of the participants. Mucosal thickenings were found in 49%, commonly in the maxillary sinuses (29%) where 25% had opacifications that were less than 4 mm in size. Other opacifications occurred in the anterior ethmoid (23%), posterior ethmoid (21%), frontal sinus (9%), and sphenoid (8%). Polyps and retention cysts were also found mainly in the maxillary sinuses in 32%. Fluid was observed in 6% of the MRIs. Mucosal thickening was observed more frequently in men than in women (P <0.05). No seasonal variation was found.

Conclusions: In this large non-selected sample, incidental opacification in the paranasal sinuses was seen in two out of three participants, and mucosal thickening was seen in one out of two. Fluid was rare. Knowledge of incidental opacification is important because it can affect clinical practice.

Background: Recent advances in magnetic resonance imaging make it possible to visualize the presumed pathophysiologic correlate of Menière's disease: endolymphatic hydrops. As traditional diagnostic tests can provide only indirect evidence, they are hardly competitive in this respect and need to be rethought. This is done here for the glycerol test.

Methods: The data of a previous retrospective analysis of the glycerol test in patients with suspected Menière's disease are reinterpreted using a simple model. The mean threshold reduction (MTR) in the frequency range from 125 to 1500 Hz (calculated from audiograms obtained immediately before and four hours after the glycerol intake) is used as the test statistic. The proposed model explains the frequency distribution of the observed MTR by the convolution of a Gaussian probability density function (representing measurement errors) with a template representing the frequency distribution of the true MTR. The latter is defined in terms of two adjustable parameters. After fitting the model to the data, the performance of the test is evaluated using receiver operating characteristic (ROC) analysis.

Results: The cumulative frequency distribution of the observed MTR can be explained almost perfectly by the model. According to the ROC analysis performed, the capability of the currently used audiometric procedure to detect a glycerol-induced threshold reduction corresponds to a diagnostic test of rather high accuracy (area under the ROC curve greater than 0.9). Simulations show that methodological improvements could further enhance the performance.

Conclusions: Owing to their ability to reveal functional aspects without an obvious morphological correlate, traditional test for Menière's disease could be decisive for defining the stage of the disease. A distinctive feature of the glycerol test is that it is capable of determining, with high accuracy, whether the pathophysiologic condition of the inner ear is partially reversible. Prospectively, this could help to estimate the chances of specific therapies.

Background: The objective of this observational study was to evaluate the outcomes of intranasal surgery in patients with obstructive sleep apnea (OSA) in a single institution in Norway.

Methods: Fifty-nine patients with OSA and clinically significant nasal obstruction underwent either septoplasty alone or septoplasty with concomitant volume reduction of the turbinates from August 2008 until the end of December 2010. Subjects were scheduled for sleep polygraphy before and 3 months after treatment. In this observational single-centre cohort study we evaluated and compared the effect of these two specific surgical procedures on sleep related parameters.

Results: There was a significant reduction in the apnea-hypopnea index (AHI) only in the group that had septoplasty with turbinate reduction (17.4, (SD 14.4) - 11.7, (SD 8.2), p <0.01), and this effect was significantly better than in the group treated with septoplasty alone. Other objective parameters remained unchanged. Subjective assessments obtained with a postoperative questionnaire showed an equally positive effect on diurnal sleepiness and nasal obstruction in both groups, and a better effect on sleep quality in the combined treatment group.

Conclusion: The effect of nasal surgery on obstructive sleep apnea seemed to be greater when there were indications for combined surgery of the inferior turbinates and the nasal septum, compared to when there were indications for septoplasty alone.

Background: Currently, there is a paucity of data concerning the long-term outcomes, educational placement and quality of life of children implanted with hearing devices from large and representative samples of the population. To address this concern, a large, prospective, multicentre, multinational patient-outcomes registry for paediatric recipients of implantable hearing devices was developed. The benefits of this registry, its approach and methodology are described.

Methods/design: The Cochlear(™) Paediatric Implanted Recipient Observational Study (Cochlear P-IROS) is a prospective international patient-outcomes registry for children who are implanted in routine clinical practice with one or more hearing devices. The study aims to collect data on patient comorbidities, device use, auditory performance, quality of life and health-related utilities, across different types of implantable hearing devices from a range of manufacturers. Patients will be evaluated with a set of standardised and non-standardised questionnaires prior to initial device activation (baseline) and at six-monthly follow-up intervals up to 24 months and annually thereafter. The Cochlear P-IROS utilises a secure web interface to administer electronic case report forms to clinicians and families of implanted children. The web interface is currently available in five languages: English, Japanese, Korean, Mandarin and Russian. The interface also provides printable versions of the case report forms translated into 22 local languages for collection of data prior to entry online; additional languages may be added, as required. Participation in the Cochlear P-IROS registry is investigator-driven and voluntary. To date, the Cochlear P-IROS has recruited implant clinics across Australia, China, India, Indonesia, Turkey and Vietnam. The registry also aims to recruit multiple clinics in Cuba, Israel, Japan, Malaysia, Singapore, South Africa, South Korea and Russia.

Discussion: The use of a registry such as the Cochlear P-IROS will generate valuable data to support research interests of academics and clinicians around the globe. The data generated will be relevant for a wide range of stakeholders including regulators, payers, providers, policy makers, patients and their families, each with a different perspective for the acceptance and adoption of implantable hearing devices for the treatment of hearing loss.

Background: Copy number variations (CNVs) are the major type of structural variation in the human genome, and are more common than DNA sequence variations in populations. CNVs are important factors for human genetic and phenotypic diversity. Many CNVs have been associated with either resistance to diseases or identified as the cause of diseases. Currently little is known about the role of CNVs in causing deafness. CNVs are currently not analyzed by conventional genetic analysis methods to study deafness. Here we detected both DNA sequence variations and CNVs affecting 80 genes known to be required for normal hearing.

Methods: Coding regions of the deafness genes were captured by a hybridization-based method and processed through the standard next-generation sequencing (NGS) protocol using the Illumina platform. Samples hybridized together in the same reaction were analyzed to obtain CNVs. A read depth based method was used to measure CNVs at the resolution of a single exon. Results were validated by the quantitative PCR (qPCR) based method.

Results: Among 79 sporadic cases clinically diagnosed with sensorineural hearing loss, we identified previously-reported disease-causing sequence mutations in 16 cases. In addition, we identified a total of 97 CNVs (72 CNV gains and 25 CNV losses) in 27 deafness genes. The CNVs included homozygous deletions which may directly give rise to deleterious effects on protein functions known to be essential for hearing, as well as heterozygous deletions and CNV gains compounded with sequence mutations in deafness genes that could potentially harm gene functions.

Conclusions: We studied how CNVs in known deafness genes may result in deafness. Data provided here served as a basis to explain how CNVs disrupt normal functions of deafness genes. These results may significantly expand our understanding about how various types of genetic mutations cause deafness in humans.

Background: Acute cervical adenitis can evolve into suppurative cervical lymphadenitis and may sometimes be associated with infection of the retropharyngeal and parapharyngeal spaces (i.e., retropharyngeal and poststyloid parapharyngeal abscesses). This study aimed to describe the clinical presentation of acute cervical lymphadenitis and infections of the retropharyngeal and parapharyngeal spaces in children and examine the management of these conditions.

Methods: This was a retrospective study including children from 3 months to 18 years old who were hospitalized in the Pediatric Department of the Centre-Intercommunal-de-Créteil between January 2003 and May 2010. Selected cases were based on the diagnosis of acute cervical lymphadenitis, suppurative cervical lymphadenitis, or infections of the retropharyngeal or parapharyngeal spaces. Case history, clinical signs, laboratory tests, imaging, treatment and clinical course were collected from patient charts.

Results: We included 75 children (54 males [72%]); 62 (83%) were < 6 years old. Diagnoses were acute cervical lymphadenitis in 43 patients (57%), suppurative cervical lymphadenitis in 13 (17%), retropharyngeal or poststyloid parapharyngeal abscess in 18 (24%) and cervical necrotizing fasciitis in 1 (1%). In total, 72 patients (96%) presented fever and 34 (45%) had torticollis. Suppurative cervical lymphadenitis or abscesses of the retropharyngeal or poststyloid parapharyngeal spaces was significantly higher for children with than without torticollis (52.9% vs. 4.8%, p < 0.001). In all, 21 patients among the 44 > 3 years old (48%) underwent a rapid antigen detection test (RADT) for group A beta-hemolytic Streptococcus pyogenes; results for 10 were positive (48%). Contrast-enhanced CT scan of the neck in children with torticollis (n = 31) demonstrated an abscess in 21 (68%). Fine-needle aspiration was performed in 8 patients (11%) and 8 (11%) required surgical drainage. Bacteriology was positive in 8 patients (11%), with a predominance of Staphylococcus aureus and S. pyogenes. All patients received intravenous antibiotics and the outcome was favorable regardless of surgery. Recurrence was observed in only 1 case among the 34 patients with a follow-up visit after discharge.

Conclusion: Our data suggest that presentation with cervical lymphadenitis associated with fever and torticollis requires evaluation by contrast-enhanced CT scan. Furthermore, abscess drainage should be restricted to the most severely affected patients who do not respond to antibiotic therapy.

Background: Individuals with a unilateral severe-to-profound hearing loss, or single-sided deafness, report difficulty with listening in many everyday situations despite having access to well-preserved acoustic hearing in one ear. The standard of care for single-sided deafness available on the UK National Health Service is a contra-lateral routing of signals hearing aid which transfers sounds from the impaired ear to the non-impaired ear. This hearing aid has been found to improve speech understanding in noise when the signal-to-noise ratio is more favourable at the impaired ear than the non-impaired ear. However, the indiscriminate routing of signals to a single ear can have detrimental effects when interfering sounds are located on the side of the impaired ear. Recent published evidence has suggested that cochlear implantation in individuals with a single-sided deafness can restore access to the binaural cues which underpin the ability to localise sounds and segregate speech from other interfering sounds.

Methods/design: The current trial was designed to assess the efficacy of cochlear implantation compared to a contra-lateral routing of signals hearing aid in restoring binaural hearing in adults with acquired single-sided deafness. Patients are assessed at baseline and after receiving a contra-lateral routing of signals hearing aid. A cochlear implant is then provided to those patients who do not receive sufficient benefit from the hearing aid. This within-subject longitudinal design reflects the expected care pathway should cochlear implantation be provided for single-sided deafness on the UK National Health Service. The primary endpoints are measures of binaural hearing at baseline, after provision of a contra-lateral routing of signals hearing aid, and after cochlear implantation. Binaural hearing is assessed in terms of the accuracy with which sounds are localised and speech is perceived in background noise. The trial is also designed to measure the impact of the interventions on hearing- and health-related quality of life.

Discussion: This multi-centre trial was designed to provide evidence for the efficacy of cochlear implantation compared to the contra-lateral routing of signals. A purpose-built sound presentation system and established measurement techniques will provide reliable and precise measures of binaural hearing.

Trial registration: Current Controlled Trials http://www.controlled-trials.com/ISRCTN33301739 (05/JUL/2013).

Background: Aim of the present study was to evaluate prognostic factors, in particular standard laboratory parameters, for better outcome after idiopathic sudden sensorineural hearing loss (SSNHL).

Methods: Using a retrospective review, 173 patients were included presenting between 2006 and 2009 with unilateral SSNHL, ≥30 dB bone conduction in three succeeding frequencies between 0.125 to 8 kHz in pure tone audiometry (PTA), and a time interval between first symptoms and diagnostics ≤ 4 weeks. Hearing gain of <10 dB versus ≥10 dB in the affected ear in 6PTA values was the primary outcome criterion. Univariate and multivariate statistical tests were used to analyze predictors for better outcome.

Results: The initial hearing loss was 50.6 ± 27.2 dB. The absolute hearing gain was 15.6 ± 20.1 dB. Eighty-one patients (47%) had a final hearing gain of ≥10 dB. Low-frequency hearing loss (p <0.0001); start of inpatient treatment <4 days after onset (p = 0.018); first SSNHL (versus recurrent SSNHL, p = 0.001); initial hearing loss ≥ 60 dB (p < 0.0001); an initial quick value lower than the reference values (p = 0.040); and a pretherapeutic hyperfibrinogenemia (p = 0.007) were significantly correlated to better outcome (≥10 dB absolute hearing gain). Multivariate analysis revealed that first SSNHL (p = 0.004), start of treatment <4 days after onset (p = 0.015), initial hearing loss ≥ 60 dB (p = 0.001), and hyperfibrinogenemia (p = 0.032) were independent prognostic factors for better hearing recovery.

Conclusion: Better hearing gain in patients with hyperfibrinogenemia might be explained by the rheological properties of the applied therapy and supports the hypothesis that SSNHL is caused in part by vascular factors.